Gastric adenocarcinoma and proximal polyposis of the stomach

From Libre Pathology
Revision as of 16:47, 16 September 2019 by Michael (talk | contribs) (+criteria)
Jump to navigation Jump to search
The printable version is no longer supported and may have rendering errors. Please update your browser bookmarks and please use the default browser print function instead.

Gastric adenocarcinoma and proximal polyposis of the stomach, abbreviated GAPPS, is a rare syndrome characterized by fundic gland polyps and gastric adenocarcinoma.[1][2]

General

  • Very rare.
  • APC promotor IB mutations.[1]

Criteria

Features - all required:[1]

  • Polyps of stomach in body and fundus only.
    • >100 polyps in index case or >30 polyps in first degree relative.
    • Histology predominantly fundic gland polyps - some with dysplasia or adenocarcinoma.
  • No colorectal or duodenal polyposis.
  • Autosomal dominant inheritance.
  • Exclusion of other heritable polyposis syndrome involving the stomach, PPI use.

See also

References

  1. 1.0 1.1 1.2 Rudloff, U. (2018). "Gastric adenocarcinoma and proximal polyposis of the stomach: diagnosis and clinical perspectives.". Clin Exp Gastroenterol 11: 447-459. doi:10.2147/CEG.S163227. PMID 30584346.
  2. Worthley, DL.; Phillips, KD.; Wayte, N.; Schrader, KA.; Healey, S.; Kaurah, P.; Shulkes, A.; Grimpen, F. et al. (May 2012). "Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS): a new autosomal dominant syndrome.". Gut 61 (5): 774-9. doi:10.1136/gutjnl-2011-300348. PMID 21813476.